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Pulmonary hypertension-"state of the art" management in 2012.

ABSTRACT: Pulmonary artery hypertension (PAH) is a pathological condition of small pulmonary arteries, characterised by vascular proliferation and remodelling. The pulmonary artery pressure and pulmonary vascular resistance progressively rise, leading to right heart failure and death. Pulmonary artery hypertension may be secondary to various conditions, or it may be idiopathic where no underlying cause is identifiable. Earlier, only symptomatic treatment was available for such patients which did not change the natural history of the disease. However, over the years, improvement in understanding the pathogenesis has resulted in the development of targeted approaches to the treatment of PAH. Survival advantage has also been shown with some of the pharmacologic agents. This review article discusses the current management strategy for PAH with special emphasis on an idiopathic variety, in an Indian context.

SUBMITTER: Saxena A

PROVIDER: S-EPMC3860802 | biostudies-other | 2012 Jan-Feb

REPOSITORIES: biostudies-other

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Pulmonary hypertension-"state of the art" management in 2012.

Saxena Anita A

Indian heart journal 20120101 1

Pulmonary artery hypertension (PAH) is a pathological condition of small pulmonary arteries, characterised by vascular proliferation and remodelling. The pulmonary artery pressure and pulmonary vascular resistance progressively rise, leading to right heart failure and death. Pulmonary artery hypertension may be secondary to various conditions, or it may be idiopathic where no underlying cause is identifiable. Earlier, only symptomatic treatment was available for such patients which did not chang ...[more]

PMID: 22572428

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Project description:Pulmonary endarterectomy is the treatment of choice for patients with operable chronic thromboembolic pulmonary hypertension (CTEPH) as it is potentially curative. In expert centers that conduct > 50 pulmonary endarterectomy procedures per year, peri- and post-surgical mortality rates are very low and long-term outcomes are excellent, with three-year post-operative survival of > 80%. Therapeutic decisions in CTEPH are based largely on the location of the arterial obstruction, with pulmonary endarterectomy for obstructions in main, lobar, and segmental vessels, and balloon pulmonary angioplasty and medical therapy for small-vessel disease. Medical therapy is also an option for patients with persistent/recurrent pulmonary hypertension after pulmonary endarterectomy or balloon pulmonary angioplasty. With increasing surgical experience and improvements in instruments and procedures, an increasing number of patients are now considered operable who would previously have been inoperable, including some patients with subsegmental disease. At our University (University of California San Diego), around 200 pulmonary endarterectomy procedures are performed every year and several advances have been developed, including resection of more distal disease, availability of pulmonary endarterectomy to patients previously considered to be at too high risk for surgery, improved management of post-pulmonary endarterectomy complications, and minimally invasive pulmonary endarterectomy. Pulmonary endarterectomy can be combined with other treatment modalities, including balloon pulmonary angioplasty, medical therapy for persistent/recurrent pulmonary hypertension after pulmonary endarterectomy, and medical therapy or balloon pulmonary angioplasty as bridging therapy before surgery. Data on these combinations are, however, limited. Combination treatment should therefore be considered on an individual patient basis. In the future, however, multimodal therapy with pulmonary endarterectomy, balloon pulmonary angioplasty, and/or medical therapy is likely to be an important treatment option for many patients.

Project description:Pulmonary hypertension is defined as a resting mean pulmonary artery pressure of 25 mm Hg or above. This review deals with pulmonary arterial hypertension (PAH), a type of pulmonary hypertension that primarily affects the pulmonary vasculature. In PAH, the pulmonary vasculature is dynamically obstructed by vasoconstriction, structurally obstructed by adverse vascular remodeling, and pathologically non-compliant as a result of vascular fibrosis and stiffening. Many cell types are abnormal in PAH, including vascular cells (endothelial cells, smooth muscle cells, and fibroblasts) and inflammatory cells. Progress has been made in identifying the causes of PAH and approving new drug therapies. A cancer-like increase in cell proliferation and resistance to apoptosis reflects acquired abnormalities of mitochondrial metabolism and dynamics. Mutations in the type II bone morphogenetic protein receptor (BMPR2) gene dramatically increase the risk of developing heritable PAH. Epigenetic dysregulation of DNA methylation, histone acetylation, and microRNAs also contributes to disease pathogenesis. Aberrant bone morphogenetic protein signaling and epigenetic dysregulation in PAH promote cell proliferation in part through induction of a Warburg mitochondrial-metabolic state of uncoupled glycolysis. Complex changes in cytokines (interleukins and tumor necrosis factor), cellular immunity (T lymphocytes, natural killer cells, macrophages), and autoantibodies suggest that PAH is, in part, an autoimmune, inflammatory disease. Obstructive pulmonary vascular remodeling in PAH increases right ventricular afterload causing right ventricular hypertrophy. In some patients, maladaptive changes in the right ventricle, including ischemia and fibrosis, reduce right ventricular function and cause right ventricular failure. Patients with PAH have dyspnea, reduced exercise capacity, exertional syncope, and premature death from right ventricular failure. PAH targeted therapies (prostaglandins, phosphodiesterase-5 inhibitors, endothelin receptor antagonists, and soluble guanylate cyclase stimulators), used alone or in combination, improve functional capacity and hemodynamics and reduce hospital admissions. However, these vasodilators do not target key features of PAH pathogenesis and have not been shown to reduce mortality, which remains about 50% at five years. This review summarizes the epidemiology, pathogenesis, diagnosis, and treatment of PAH.

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Pulmonary hypertension-"state of the art" management in 2012.

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Pulmonary hypertension-"state of the art" management in 2012.

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